Radiographic and histologic findings in Paget\u27s disease of bone have been previously well described, and characteristic features of each stage have been determined. Long-term radiographic follow-up of untreated patients is rarely reported. We had the opportunity to observe the radiographic evolution of untreated Paget\u27s disease of the tibia. Sequential radiographic studies spanning a 19-year period have allowed us the opportunity to study the natural course of this disease

Ellis, Burton I.

Kleerekoper, Michael

Shier, Christopher K.

Tennyson, Gregory

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Henry Ford Hospital Medical Journal Volume 34 Number 2 Article 15 6-1986 Nineteen-Year Radiographic Follow-up of Untreated Paget's Disease of Bone Christopher K. Shier Burton I. Ellis Gregory Tennyson Michael Kleerekoper Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Shier, Christopher K.; Ellis, Burton I.; Tennyson, Gregory; and Kleerekoper, Michael (1986) "Nineteen-Year Radiographic Follow-up of Untreated Paget's Disease of Bone," Henry Ford Hospital Medical Journal : Vol. 34 : No. 2 , 127-129. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol34/iss2/15 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. IG. D- pen-y in one pa-itis: A case 5 in D-pen-7-30. yositis: Ob-•amre. Ami if peripheral halothane in Engl J Med toid disease: ;s. Br MedJ n KW. Pen-and genetic I in Japanese). 1 rheumatoid Î ineteen-Year Radiographic Follow-up of Untreated Paget's pisease of Bone Christopher K. Shier, MD,* Burton I . Ellis, MD,* Gregory Tennyson, MD,^ and IVIichael Kleerekoper, MD^ Radiographic and histologic findings in Paget's disease of bone have been previously well described, and characteristic features of each stage have been determined. Long-term radiographic follow-up of untreated patients is rarely reported. We had the opportunity to observe the radiographic evolution of untreated Paget's disease ofthe tibia. Sequential radiographic studies spanning a 19-year period have allowed us the opportunity to study the natural course of this disease. (Henry Ford Hosp Med J 1986;34:127-9) Case Report A63-year-old man was initially seen at Henry Ford Hospital in 1963 because of increasing shin pain which had been present for several months. At that time a diagnosis of Paget's disease was made, and a brief trial of aspirin therapy was instituted. The patient's anterior tibial pain persisted and worsened with prolonged use of the leg. The patient experienced periods both of exacerbation and remission of symptoms, but over the last two decades no further therapy was deemed necessary by him. In August 1985, calcitonin therapy was instituted after several nionths of right ankle pain. The patient reported separate instances of trauma to the right leg caused by falls in 1968, 1974, and 1980. He also described "sensitivity to kneeling," with persistent pain in the knees and occasional brief "locking" for the past several years. He has a past medical history of gout and essential hypertension. Physical examina-tion revealed moderate deformity ofthe right leg and slight warmth over the tibia. Laboratory investigation revealed an alkaline phosphatase of 1.033 lU/L; past measurements had ranged from 200 to 300 lU/L. Radiographic evaluation was obtained intermittently between 1966 and 1985. In 1966, roentgenograms of the right tibia revealed a well-defined radiolucency involving the anterior cortex in the proximal iliametaphy.seal region, with a characteristic V-shaped distal margin. No sclerosis, cortical thickening, periosteal reaction, or soft tissue swelling was evident. The lytic region was seen to spare the proximal end of the tibia (Fig lA). In 1972, the lesion had progressed distally to the middiaphysis, with •minimal proximal extension. At this time, the border between normal and pagetoid bone was less well-defined than in 1966. Smudging and (hoarsening of the trabecular pattern within the region of abnormality *ere evident, along with irregular periosteal cortical thickening (Fig IB). In 1980, further progression to the distal tibial diametaphyseal region *as observed. Radiographs revealed marked undulent cortical thicken-"•g throughout the tibial diaphysis, with regions of lysis and sclerosis "nd disorganization of the trabecular pattem. Ill-defined sclerosis was '̂ en in formerly lytic zones proximally, with predominantly lytic in-volvement of the more recently affected distal tibia (Fig IC). On radiographic evaluation in 1985, further distal progression of the changes were evident. Cortical thickening and trabecular coarsening ^̂ fe combined with poorly marginated patches of sclerosis. A large bony spur superior to the tibial tubercle also demonstrated findings of Paget's disease. The changes appeared to extend to both the proximal and distal articular surfaces of the tibia (Fig ID). Radionuclide bone scan demonstrated intense increased uptake throughout the entire length of the right tibia (Fig 2). Increased uptake was also evident in the left hemipelvis, proximal left femur, and right humems. Classic radiographic changes of Paget's disease were seen in these latter locations, with evidence for previous pathologic fracture through the distal right humerus. Discussion Osteitis deformans was first described by Sir James Paget in 1877 (1). This bone disease generally has its onset in mid-adult life and increases in incidence with age, although cases have oc-curred in young adults (2). The pathologic changes are com-monly divided into three phases: initial , active, and inactive (3,4). The early phase is characterized roentgenographically by bone resorption, appearing classically as a radiolucent area with a V-shaped distal margin involving both cortical and cancellous bone. This initial phase corresponds to the so-called "flame" or "blade of grass" appearance, with a sharply defined oblique border between normal and abnormal bone (4). The term os-teoporosis circumscripta is used to describe the appearance of this early phase in flat bones such as the calvarium. Intense increased uptake is usually seen in active Paget's disease on radionuclide bone scan, although areas of photopenia have been postulated as possible precursors of active Paget's disease (5). The findings on the initial radiographs in our patient demon-strate the characteristic appearance of the lytic phase of the dis-ease. At this point, biopsy usually shows intense osteoclastic Submitted for publication; March 25, 1986. Accepted for publication: April 14, 1986. ^Department of Diagnostic Radiology, Henry Ford Hospital. tDivision of Bone and Mineral Metabolism, Henry Ford Hospital. Address correspondence to Dr Shier, Department of Diagnostic Radiology, Henry Ford Hospital, 2799 W Grand Blvd, Detroit. Ml 48202. Matsumura etal, He, "fy Ford Hosp Med J—Vol 34, No 2, 1986 Untreated Paget's Bone Disease—Shier et al 127 (A) (B) (C) (D) Fig I—Lateral radiographs of the tibia spanning a 19-year period. (A) 1966: Note the characteristic radiolucent area with a sharply defined oblique inferior margin (arrows). (B) 1972: Progression of resorption distally. with less well-defined border between normal and pagetoid bone. (C) 1980: Fur-ther distal progression of lysis, with disorganization ofthe trabecular pattern and marked periosteal new bone (arrows). (D) 1985: Extension to involve the entire tibia. Note the increased size and anterior bowing ofthe tibia. KNEES TIBIAE Fig2—Isotope bone scan (1985). Markedly increased uptake throughout the entire length ofthe right tibia, indicating active disease. activity at the junction between normal and abnormal bone. Ar-chitecturally disorganized osteoblastic activity is responsible for irregular pagetoid bone adjacent to the junction region (4). The radiographic progression of the active phase of Paget's disease can be observed in our patient. Lytic resorption of bone was seen to progress gradually throughout the distal tibial diaphysis. At any point in time, the more recently affected re-gions of bone demonstrate predominant lysis, with increasing patchy sclerosis affecting the earlier involved proximal portion of the tibia. Advancement from one end of a long bone to the other is characteristic, although mid-diaphyseal presentation in the tibia has been reported (6,7). Disease progression within a bone is indicated by advancement of the resorption front, devel-opment of new resorption clefts, and increase in bone size with cortical thickening. Conversely, a decrease in bone size and improved corticomedullary differentiation with a decrease in cortical thickening are characteristic of disease regression (8)-128 Henry Ford Hosp Med J—Vol 34. No 2, 1986 Untreated Paget's Bone Disease—Shier et al I I j^gijiineralization is also seen, with gross bowing deformities re-sulting from the extensive remodeling and weakening of bone. pathologically, the new pagetoid bone develops in an irregular pattern in contrast to the organized longitudinal osteon orienta-tion of normal bone. Hypervascularity is prominent within the bone marrow, with loose fibrotic changes (4). A wide zone of spongy periosteal bone, which has been likened to pumice, may form around the original cortex (9). Similarly, endosteal new bone may also cause cortical thickening, although this is less frequently recognized (3,4). Ultimately, the remodeling, with constant interplay between resorption and new bone formation, results in the classic histologic mosaic pattem. At the most re-cent evaluation, the disease in our patient was still active, as evi-denced by symptoms and the findings on isotope bone scan. The inactive phase is reached after many years, with reduc-tion of abnormal osteoblastic and osteoclastic activity. Radio-graphically, the changes of inactive Paget's disease may be difficult to differentiate from advanced active disease (4); hence, the value of the isotope bone scan. Deformities of an affected bone may lead to malalignment of joints with secondary os-teoarthritic changes. Basilar invagination of the skull, spinal stenosis, and stress fracture, usually in the later stages, may oc-cur. Sarcomatous degeneration is a well-documented late com-plication (10). Drugs commonly used to treat Paget's disease, such as cal-citonin and mithramycin, appear to produce remission by caus-ing an inhibition of excessive osteoblastic and osteoclastic cellular activity (11-14). Following the recent exacerbation of symptoms at the distal right tibia, calcitonin therapy was initi-ated in our patient. Summary Although the radiographic and pathologic changes in the three commonly recognized phases of Paget's disease of bone have been well described, they are rarely observed over a long period of time in untreated patients. Our report of a patient, who was followed clinically and roentgenographically for 19 years, has allowed us the unusual opportunity to study the natural pro-gression of this disease. References 1. Paget J. On a form of chronic inflammation of bones. Med Chir Trans (London) 1877;60:37-64. 2. Greenspan A, Norman A, Sterling AP. Precocious onset of Paget's dis-ease—a report of three cases and review of the literature. J Can Assoc Radiol 1977;28:69-72. 3. Jaffe HL. Paget's disease of bone. Arch Pathol 1933;15;83-131. 4. Milgram JW. Radiological and pathological assessment of the activity of Paget's disease of bone. Clin Orthop 1977;127:43-54. 5. Jaspers MM, Pauwells EK, Harinck HI, Blom J. Photon-deficient bone scan lesion as a precursor of active Paget's disease. Diagn Imag Clin Med 1984;53:144-8. 6. Schlesinger A, Naimark A, Lee VW. Diaphyseal presentation of Paget's disease in long bones. Radiology 1983;147:83-4. 7. Zadek RE, Milgram JW. Progression of Paget's disease in the tibia. A case report. J Bone Joint Surg lAm] 1976;58A:876-8. 8. Woodhouse NJY, Chalmers AH, Wells IP, Dewbury KC, Mohamedally SM. Paget's disease. Radiological changes occurring in untreated patients and those on therapy with salmon calcitonin during two years' observation. BrJ Radiol 1977;50:699-705. 9. Johnson LC. Morphological analysis in pathology: The kinetics of disease and general biology of bone. In: Frost HM, ed. Bone Biodynamics. Boston; Lit-tle, Brown and Co, 1964:595-9. 10. Wick MR, Siegal GP Unni KK, McLeod RA, Greditzer HG. Sarcomas of bone complicating osteitis deformans (Paget's disease). Fifty years' experi-ence. Am J Surg Pathol 1981;5:47-59. 11. Condon JR, Reith SBM, Nassim JR, Millard FJC, Hilb A, Stainthorpe EM. Treatment of Paget's disease of bone with mithramycin. Br Med J IClin Res] 1971;l;42l-3. 12. Elias EG, Evans JT. Mithramycin in the treatment of Paget's disease of bone. J Bone Joint Surg lAm] 1972;54A; 1730-6. 13. Bijvoet OL, Van der Sluys Veer J, Jansen AP. Effects of calcitonin on patients with Paget's disease, thyrotoxicosis, or hypercalcemia. Lancet 1968;1:876-81. 14. Johnston CC. Calcitonin in die treatment of Paget's disease of bone (Edi-torial note). Ann Intern Med 1972;76:665-6. He, "'y Ford Hosp Med J—Vol 34, No 2, 1986 Untreated Paget's Bone Disea.se—Shier et al 129 

Nineteen-Year Radiographic Follow-up of Untreated Paget\u27s Disease of Bone

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